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August 13, 2010

A most unexpected talk with neurology

Monday, I went into Bertrand's neurology follow-up feeling conflicted. Bertrand has made amazing progress the past 2 months. He can stand with assistance, he can use his left arm, he can stay on hands and knees for a minute or two if placed there, he can sit without falling over, he is more social than ever, he is eating solid foods, he is making consonant sounds... but he is also seizing again.

Starting a few weeks ago, his myoclonus started making appearances throughout the day and his complex partial seizures show up toward the end of the day. I was optimistic but preparing myself to be terrified by what I saw on his latest EEG.

Unfortunately, I couldn't make heads or tails of Bertrand's EEG! Despite being slightly sleep deprived Bertrand didn't fall into his usual EEG trance. Oh, no! Bertrand was literally bouncing off the walls of his hospital bed! He was rolling back and forth, propping himself up on his forearms, talking at me (some cursing, some pleading, some flirting), trying to look at the computer monitor, playing with the blankets and the bed itself, reaching for toys... and he hadno myoclonus during the session. Of course, he had them before and after, just not during. Ugh!

A moving baby's EEG looks very different from a sleeping baby's EEG. I saw lots of crazy lines but only a handful of spikes. It killed me that I'd have to wait until speaking with Bertrand's neurologist to draw a conclusion. But, what a conclusion it was!

Bertrand's neurologist walked in and said, "if you hadn't told me this was Bertrand's EEG, I would've assumed it was a normal child with a predisposition for seizures. There is no way I would've thought this was a child with a seizure disorder!" The news got even better, as she admitted that she would've never thought Bertrand's EEG could ever look this good and that she was now a "convert" to ACTH and steroids.

However, during the session with the neurologist, even she noted Bertrand's myoclonus and "shakes". She believes that his shaking and possibly even some of the myoclonus could simply be Bertrand's movement disorder, but they look like seizures. To be safe rather than sorry, Bertrand was started on zonegran, a medication best suited for myoclonic seizures.

I have several issues with dismissing his abnormal movements as a movement disorder:

Those abnormal movements disappeared on the ketogenic diet, a treatment for epilepsy.

Those abnormal movements are frequently followed by disorientation, confusion or staring, which are indicative of seizure activity rather than movement disorder.

Occam's Razor: "entities must not be multiplied beyond necessity" (entia non sunt multiplicanda praeter necessitatem). When competing hypotheses are equal in other respects, the principle recommends selection of the hypothesis that introduces the fewest assumptions and postulates the fewest entities while still sufficiently answering the question. This means, why assume a child has both a seizure and a movement disorder if just a seizure disorder could sufficiently describe his condition? I am starting to believe that Bertrand just had epilepsy from the get-go and, because some of his seizures (shakes, jerks, and automatisms) resemble movement disorders, we were led on a wild goose chase.

While at the doctor's appointment, we discussed the possibility of restarting the ketogenic diet, but the consensus was that if Bertrand's seizures could be effectively treated by adding one other medication (zonegran), with minimal side-effects, that would be best. The ketogenic diet combined with the steroids and Bertrand's lack of sufficient weight-bearing poses a very serious risk to his already weak bones. (Last February's broken arm set Bertrand back bymonths developmentally AND made him miserable.)

Unfortunately, the rest of this week has gone horribly for Bertrand. In spite of starting the zonegran on Monday, Bertrand shakes and myoclonus have gotten increasingly frequent and violent. His social awareness is slipping and his irritability/fogginess is rolling back in. Matthew and I are already mourning the loss of our son's personality. Granted Bertrand still has 5 more weeks to achieve an effective level on zonegran in his blood stream, but at the rate his seizure control is slipping, that is 5 weeks too many.

Today, I called the nurse practitioner in-charge of the ketogenic diet at Primary Children's Medical Center to see how soon we could go back on the diet. The ketogenic diet worked immediately by eliminating Bertrand's atonic seizures and reducing his myoclonus to at most one per day. But Bertrand can't restart the diet for another 2 months at the earliest.

I want to be happy about what Bertrand has achieved so far, but I am devastated. Bertrand is already slipping away and the next stage of the prednisolone wean is supposed to start Monday. How can I avoid losing my baby? Do we start the modified atkins diet, doctors be damned? Do we wait to continue the prenisolone wean? Do we increase or reduce one of his medications (keppra, zonegran)? You should never change multiple variables at once but the temptation is almost impossible to resist when your child's welfare is at stake.

Wow. I understand how you don't want to lose Bertrands new skills, they are amazing. Have you tried going off all the drugs (in stages) until only the diet is left? Especially after his ACTH treatment. Why would he have to wait for two months? It's always tricky when the doc says more meds when the current ones aren't working. I am sure you will make a good decision. At some point it is you that will have to tell the doctor what to do, after all he/she is working for you. My thoughts are with your family everyday. You guys will make it through this too.

EEG biofeedback could possibly be used in conjunction with the iPad Bertrand's been using for some seizure reduction. Might be some computer program(s) available for use with a child who doesn't have good receptive communication.

Good review on EEG neurofeedback with epilepsy by Sterman and Egner, 2006: "Foundation and practice of neurofeedback for the treatment of epilepsy".

Might try contacting someone certified in EEG biofeedback for consultation help as for applicability. Worth a shot?

Anonymous: I am very interested in biofeedback and will look into it! Thanks for reminding me about it.

Amy and Barbara: Tough decisions indeed! Thank you both for your consistent support and calming influence. :)

I think we will postpone lowering Bertrand's prednisolone for at least another week (if not another month) and just increase the zonegran on schedule. That way we are only changing one thing at a time. We'll also start looking into the modified atkins diet in the interim and may begin shifting toward it.

Oh my goodness, I can't even begin to imagine having to juggle all of that - talk about the devil's arithmetic. We're thinking about you and hoping everything works out for the best and that the zonegran can keep him in good shape while keeping his personality unveiled, so to speak. (Incidentally, Daniel still talks about Bertrand quite a bit - he insists that Veronica wants to be a bee for Halloween this year, because Bertrand was a bee last year. I've been telling him that Halloween is more than two months away so let's not stress about it yet).

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